9679/3 Mediastinal (thymic) large B-cell lymphoma


Synonyms

Thymic large B-cell lymphoma

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C85.2
           

/span> diffuse large B-cell lymphoma arising in the mediastinum from putative thymic B-cell origin with distinctive clinical, immuno?phenotypic and genotypic features.


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Thymus
ICD-O-3 topography code: C37
ICD10: C85.2
           

Primary mediastinal large B-cell lymphoma (PMBL) is a non-Hodgkin lymphoma of the mediastinum, probably arising in the thymus. InvasioH cells in all NLPHL cases(116)
A20 and TRAF1 are two anti-apoptotic components of the intracellular signaling pathway of the tumor necrosis factor receptor (TNFR) family. CD30 stimulation induces A20 and TRAF1 expression.(117) A20 but not TRAF1 is expressed in NLPHL cases, although in variable numbers of Ln of lungs, pleura or pericardium is frequent as is metastatic spread in the course of disease progression. PMBL is histologically characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis.
PMBL accounts to 2-4% of non-Hodgkin lymphomas and manisfests preferentially in young adults, with a marked female predominance
3
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



. Response to intensive chemotherapy, with or without radiotherapy, is usually good and clinical outcome of PMBL patients is considered equivalent or superior to that of other patients with diffuse large B-cell lymphoma (DLBCL)4
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.



Mediastinum
ICD-O-3 topography code: C38.1-C38.3
ICD10: C85.2
           

Primary mediastinal large B-cell lymphoma (PMBL) is a non-Hodgkin lymphoma of the mediastinum, probably arising in the thymus. InvasioH cells in all NLPHL cases(116)
A20 and TRAF1 are two anti-apoptotic components of the intracellular signaling pathway of the tumor necrosis factor receptor (TNFR) family. CD30 stimulation induces A20 and TRAF1 expression.(117) A20 but not TRAF1 is expressed in NLPHL cases, although in variable numbers of Ln of lungs, pleura or pericardium is frequent as is metastatic spread in the course of disease progression. PMBL is histologically characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis.
PMBL accounts to 2-4% of non-Hodgkin lymphomas and manisfests preferentially in young adults, with a marked female predominance
5
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



. Response to intensive chemotherapy, with or without radiotherapy, is usually good and clinical outcome of PMBL patients is considered equivalent or superior to that of other patients with diffuse large B-cell lymphoma (DLBCL)6
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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